Continuum (Minneap Minn). 2020 Oct;26(5):1184-1204. doi: 10.1212/CON.0000000000000929.
This article reviews the clinical features, diagnosis and differential diagnosis, prognosis, pathogenesis, and current and upcoming treatments of Guillain-Barré syndrome (GBS).
GBS is an acute inflammatory neuropathic illness with striking clinical manifestations and significant morbidity. A substantial proportion of patients with GBS do not respond to current immunomodulatory therapies (ie, plasma exchange and IV immunoglobulin [IVIg]), highlighting the need for new therapies. Prognostic models that can accurately predict functional recovery and the need for artificial ventilation have emerged. These models are practical, and online calculators are available for clinical use, facilitating early recognition of patients with poor outcome and the opportunity to personalize management decisions. Clinical and experimental studies have identified innate immune effectors (complement, macrophage lineage cells, and activating Fcγ receptors) as important mediators of inflammatory nerve injury. Two complement inhibitors are undergoing clinical testing for efficacy in GBS.
GBS is the most common cause of acute flaccid paralysis in the United States and worldwide. New treatments for GBS have not emerged since the 1990s. Our understanding of the pathogenesis of this disorder has progressed, particularly over the past decade; as a result, new therapeutic agents targeting different components of the complement cascade are at advanced stages of clinical development.
本文综述了吉兰-巴雷综合征（GBS）的临床特征、诊断与鉴别诊断、预后、发病机制，以及目前和即将出现的治疗方法。
GBS 是一种急性炎症性脱髓鞘性神经病，具有显著的临床表现和较高的发病率。相当一部分 GBS 患者对目前的免疫调节治疗（即血浆置换和静脉注射免疫球蛋白[IVIg]）无反应，这凸显了新疗法的必要性。能够准确预测功能恢复和需要人工通气的预后模型已经出现。这些模型实用，并且有在线计算器可供临床使用，有助于早期识别预后不良的患者，并为个性化治疗决策提供机会。临床和实验研究已经确定了固有免疫效应物（补体、巨噬细胞谱系细胞和激活的 Fcγ 受体）是炎症性神经损伤的重要介质。两种补体抑制剂正在进行临床试验，以评估其在 GBS 中的疗效。
GBS 是美国和全球范围内急性弛缓性瘫痪的最常见原因。自 20 世纪 90 年代以来，GBS 尚无新的治疗方法问世。我们对这种疾病发病机制的认识，特别是在过去十年中，已经取得了进展；因此，针对补体级联不同成分的新型治疗药物已经处于临床开发的后期阶段。
